


My Disability

by Chiari_girl



Category: Original Work
Genre: Chiari, Disability, Gen
Language: English
Status: Completed
Published: 2013-08-08
Updated: 2013-08-08
Packaged: 2017-12-22 20:32:02
Rating: General Audiences
Warnings: Creator Chose Not To Use Archive Warnings
Chapters: 1
Words: 1,411
Publisher: archiveofourown.org
Story URL: https://archiveofourown.org/works/917723
Author URL: https://archiveofourown.org/users/Chiari_girl/pseuds/Chiari_girl
Summary: <blockquote class="userstuff">
      <p>This is also posted on another website quotev it's my original story and not stolen.<br/>Please leave a comment on what you think.</p>
    </blockquote>





	My Disability

**Author's Note:**

> This is also posted on another website quotev it's my original story and not stolen.  
> Please leave a comment on what you think.

I started school like a normal child. Did normal things like irish dancing and was just myself. Nothing could prepare me for what was going to happen. This is my story.

Being a healthy child you would not think of having back pain. My parents thought I had pulled a musle while dancing but they were so wrong. I first went to my G.P and she notised that i had some curves in my lower spine where i had my pain. I was refered for x-rays and was on a waiting list. During that time I got progressivly (s.p) worse I started to loose feeling down my right side and was sent for a emergency m.r.i scan at the hospital. I could tell there was something wrong but it was not what I was expecting.

I was diagnosed with a rare brain disorder called Arnold Chiari Malformation Type 1 and Syringomyilia, M.E and Cronic pain syndrome complex. This is some info on what A.C.M is.

 

 

Arnold–Chiari malformation, or often simply Chiari malformation, is a malformation of thebrain. It consists of a downward displacement of the cerebellar tonsils through the foramen magnum (the opening at the base of the skull), sometimes causing non-communicating ]hydrocephalus as a result of obstruction of cerebrospinal fluid (CSF) outflow. The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in thevasculature of the brain. It can cause headaches, fatigue, muscle weakness in the head and face, difficulty swallowing, dizziness, nausea, impaired coordination, and, in severe cases, paralysis.

 

 

The Austrian pathologist Hans Chiari in the late 19th century described seemingly related anomalies of the hindbrain, the so called Chiari malformations I, II and III. Later, other investigators added a fourth (Chiari IV) malformation. The scale of severity is rated I - IV, with IV being the most severe. Types III and IV are very rare.

Type Presentation Other notes  
I A congenital malformation. Is generally asymptomatic during childhood, but often manifests with headaches and cerebellar symptoms. Herniation of cerebellar tonsils. The most common form.  
Syndrome of occipitoatlantoaxial hypermobility An acquired Chiari I Malformation in patients with hereditary disorders of connective tissue. Patients who exhibit extreme joint hypermobility and connective tissue weakness as a result of Ehlers-Danlos syndromeor Marfan Syndrome are susceptible to instabilities of the craniocervical junction and thus acquiring a Chiari Malformation. This type is difficult to diagnose and treat.    
II Usually accompanied by a lumbar myelomeningocele leading to partial or complete paralysis below the spinal defect. As opposed to the less pronounced tonsillar herniation seen with Chiari I, there is a larger cerebellar vermian displacement. Low lying torcular herophili, tectal beaking, and hydrocephalus with consequent clival hypoplasia are classic anatomic associations. The position of the torcular herophili is important for distinction from Dandy-Walker syndrome in which it is classically upturned. This is important because the hypoplastic cerebellum of Dandy-Walker may be difficult to distinguish from a Chiari malformation that has herniated or is ectopic on imaging. Colpocephaly may be seen due to the associated neural tube defect.    
III Causes severe neurological defects. It is associated with an occipital encephalocele.    
IV Characterized by a lack of cerebellar development.    
Other conditions sometimes associated with Chiari Malformation include hydrocephalus, syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders such as Ehlers-Danlos syndrome and Marfan Syndrome.

Chiari malformation is the most frequently used term for these types of malformations. The use of the term Arnold–Chiari malformation has fallen somewhat out of favor over time, although it is used to refer to the type II malformation. Current sources use "Chiari malformation" to describe four specific types of the condition, reserving the term "Arnold-Chiari" for type II only.. Some sources still use "Arnold-Chiari" for all four types. This article uses the latter convention.

Chiari malformation or Arnold–Chiari malformation should not be confused with Budd-Chiari syndrome, a hepatic condition also named forHans Chiari.

 

Symptoms

Headaches aggravated by Valsalva maneuvers, such as yawning, laughing, crying, coughing, sneezing or straining  
Tinnitus (ringing in the ears)  
Dizziness and vertigo  
Nausea  
Nystagmus (irregular eye movements)  
Facial pain  
Muscle weakness  
Impaired gag reflex  
Restless Leg Syndrome  
Sleep Apnea  
Dysphagia (difficulty swallowing)  
Impaired coordination  
intercranial pressure  
pupillary dilatation  
Dysautonomia: tachycardia (rapid heart), syncope (fainting), polydipsia (extreme thirst), chronic fatigue  
The blockage of Cerebro-Spinal Fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia. Central cord symptoms such as hand weakness, dissociated sensory loss, and, in severe cases, paralysis may occur.

 

Once symptomatic onset occurs, a common treatment is decompression surgery, in which a neurosurgeon usually removes the lamina of the first and sometimes the second or even third cervical vertebrae and part of the occipital bone of the skull to relieve pressure. The flow of spinal fluid may be accompanied by a shunt. Since this surgery usually involves the opening of the dura mater and the expansion of the space beneath, a dural graft is usually applied to cover the expanded posterior fossa.

A small number of neurological surgeons believe that detethering the spinal cord as an alternate approach relieves the compression of the brain against the skull opening (foramen magnum), obviating the need for decompression surgery and associated trauma. However, this approach is significantly less documented in the medical literature, with reports on only a handful of patients. It should be noted that the alternative spinal surgery is also not without risk.[citation needed]

On April 24, 2009, a young patient with Type 1 Chiari malformation was successfully treated with a minimally invasive endoscopic transnasal procedure by Dr. Richard Anderson at the Columbia University Medical Center Department of Neurosurgery.

Prognosis

The prognosis differs dependent on the type of malformation (i.e., type I, II, III, or IV). Type I is generally adult-onset and, while not curable, is treatable and rarely fatal. Syndrome of Occipitoatlantoaxial Hypermobility (Ehlers-Danlos syndrome related) is more difficult to treat than the congenital form of the disease. Individuals with this type do not respond well to the decompression surgery and often require an occipitoatlantoaxial fusion for stability. These patients are at risk of experiencing serious heart complications. Types I and II sufferers may also develop syringomyelia. Type II is typically diagnosed at birth or prenatally. Approximately 33% of individuals with Chiari II malformation develop symptoms of brainstem damage within five years; a 1996 study found a mortality rate of 33% or more among symptomatic patients, with death frequently occurring due to respiratory failure. 15% of individuals with Chiari II malformation die within two years of birth. Among children under two who also have myelomeningocele, it is the leading cause of death. Prognosis among children with Chiari II malformation who do not have spina bifida is linked to specific symptoms; the condition may be fatal among symptomatic children when it leads to neurological deterioration, but surgical intervention has shown promise. Types III and IV are extremely rare and patients generally do not survive past the age of two or three.

 

History

 

An Austrian pathologist, Hans Chiari, first described these hindbrain malformations in the 1890s. A colleague of Professor Chiari, Dr. Julius Arnold, later contributed to the definition of the condition, and students of Dr. Arnold (Schwalbe and Gredig) suggested the term "Arnold-Chiari malformation" to henceforth refer to the condition.

Some sources credit the characterization of the condition to Cleland or Cruveilhier.

I was told i would be heading to hospital in a week to have a posterior fossa  decompression. My whole life was turned upside down, going into hospital with back pain and comming out with a rare birth defect. Life would never be the same again. People feel sorry for me but I don't want that I Just want to be a normal teenager who does irish dancing and goes to school like anyone else but I wouldn't be able to do that again. But feeling sorry for my self would not help I just had to get through the week and have my surgury. I was keeped in the h.d unit of the childrens ward and even had to attened the school. After Surgury I was very Sick. Things became harder for me like walking,wrighting and everyday things people thake for granted. Life got worse when i started to get headaches it is the worst pain imagnable. Me being positive and my best friends and family have got me through this time and now twoyear later and this is where im at.

 

 


End file.
